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cardio.acute-hf.hemochromatosis-iron-overload.v1PRODUCTION
cardio.acute-hf.hemochromatosis-iron-overload.v1

Acute HF — hemochromatosis / iron-overload cardiomyopathy

cardiologyacuteadult
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Cardiac iron-overload cardiomyopathy decompensation: HH (HFE C282Y homozygote ~80%) or transfusional (chronic transfusion-dependent); restrictive then dilated phenotype; conduction system involvement; cardiac MRI T2* gold standard; phlebotomy (HH) or chelation (transfusion-dependent or HH-intolerant); GDMT 4-pillar standard

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iron-overload cardiomyopathy framed

Patient inputs (16)

C282Y homozygote (pY282Y) is most common HH genotype (~80% of HH); compound heterozygote (C282Y/H63D) lower penetrance; H63D homozygote rarely causes clinically significant overload; non-HFE HH if iron overload + negative HFE genotype

GOLD STANDARD for cardiac iron quantification per Anderson PMID 11713075 + Pennell PMID 16735649; T2* <20 ms = iron loading; <10 ms = severe; <6 ms = imminent HF risk; quantitatively guides chelation intensity

HH typically presents 30-50 in men (faster iron accumulation absent menstrual losses); 50-70 in women (post-menopause); transfusional iron-overload across ages depending on transfusion duration

Transfusion-dependent disorders (β-thalassemia major, sickle cell, MDS, aplastic anemia, hereditary spherocytosis); 20+ lifetime units pushes iron load past threshold; lifetime burden quantified in mg iron (~200-250 mg/unit pRBC)

Ferritin >1000 ng/mL is threshold for significant iron overload (per EASL 2022 + AASLD 2011); >2500 portends end-organ damage; note ferritin is acute-phase reactant — interpret with CRP

Transferrin saturation >45% (women) or >50% (men) is screening threshold; >50% with elevated ferritin = HH likely; >70% in advanced disease

Anemia screen (transfusion-dependent disorders); cytopenias may limit chelation tolerance; baseline before chelation initiation (deferiprone agranulocytosis monitoring)

LFT elevation suggests hepatic iron deposition + cirrhosis; eGFR for diuretic + chelator dosing (deferasirox renal monitoring); albumin for nutritional + cirrhosis assessment

Marker of HF severity; trend during admission for response to diuresis; NT-proBNP often markedly elevated in iron-overload cardiomyopathy

Restrictive then dilated phenotype; LV/RV dysfunction; conduction system involvement (AV block); pericardial effusion screen; serial echo for chelation response

Conduction system iron deposition (AV block, sick sinus, intraventricular block); arrhythmia (AF common); low voltage in restrictive phase; serial ECG q3mo for conduction surveillance

SBP <90 + lactate ≥2 + cool extremities = SCAI C+ shock → ROUTE to shock dossier; this engine handles SCAI A-B (warm + wet) only

Liver iron concentration (LIC) by MRI R2 (FerriScan); >7 mg/g dry weight = significant overload; >15 mg/g = severe; complements cardiac T2* for total body iron burden assessment

Men present earlier with HH (~10-20 years younger than women due to absent menstrual iron losses); women often spared until post-menopause; transfusional iron-overload sex-independent

HFE C282Y homozygote prevalence ~1:200-1:400 in Northern European descent (highest in Irish, Scottish); much lower in non-European populations; non-HFE hemochromatosis can occur in any population

HH is autosomal recessive — first-degree relatives have 25% homozygote risk; family history of unexplained cirrhosis, DM, arthropathy, or HF should trigger HH workup

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (6)

6 need judgement
  • informationallife_threateningsevere_cardiac_iron_overload_t2star_below_6ms_imminent_hf
    Cardiac MRI T2* <6 ms — imminent HF risk per Anderson PMID 11713075 + Wood PMID 18353963; aggressive combination chelation required (deferiprone + deferoxamine per Tanner PMID 17389272 TIC trials)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningprogression_to_cardiogenic_shock_iron_overload
    Hemodynamic deterioration to SCAI C+ shock physiology (SBP <90 + lactate ≥2 + cool extremities + organ dysfunction) — iron-overload cardiomyopathy progression to shock
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateninghigh_grade_av_block_from_iron_deposition
    High-grade AV block (Mobitz II, complete heart block) ± syncope from iron-deposition conduction system disease
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningdeferiprone_agranulocytosis
    ANC <1500 OR severe neutropenia during deferiprone therapy (1-2% incidence)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveredeferasirox_renal_or_hepatic_toxicity
    Rising creatinine (>33% from baseline OR Cr >0.3 above baseline) OR AST/ALT >5x ULN during deferasirox therapy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveretransplant_listing_decision_for_persistent_severe_dysfunction
    Persistent severe LV dysfunction (EF <25) at 6+ mo despite optimized GDMT + iron removal (chelation or phlebotomy) → transplant listing eligibility decision
    Trigger could not be auto-evaluated — needs clinician judgement.

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Recommended regimen

Iron-overload cardiomyopathy ADHF — supportive ADHF + GDMT 4-pillar + PHLEBOTOMY (HH) or IRON CHELATION (transfusion-dependent or HH-intolerant); coordinate with hematology + hepatology + endocrinology (EASL 2022 PMID 36064151; AASLD 2011 PMID 21452290; EHA-EBMT 2022 PMID 35139194; Anderson PMID 11713075)
axis: iron_overload_cardiomyopathy_phenotype
Selected axis "Iron-overload cardiomyopathy ADHF — supportive ADHF + GDMT 4-pillar + PHLEBOTOMY (HH) or IRON CHELATION (transfusion-dependent or HH-intolerant); coordinate with hematology + hepatology + endocrinology (EASL 2022 PMID 36064151; AASLD 2011 PMID 21452290; EHA-EBMT 2022 PMID 35139194; Anderson PMID 11713075)" by default fallback (first axis)
  • furosemide
    first line
    loop_diuretic
    40-80 mg IV (diuretic-naive); 2.5x outpatient PO dose IV if on chronic loop (DOSE-trial guided) • IV • q12h titrate
    triggers: volume_overload_with_pulmonary_or_systemic_congestion
    DOSE PMID 21366472 — high-dose IV bolus or continuous infusion equivalent; titrate to UOP + symptom resolution; transition to PO before discharge
    rxcui 4603
  • nitroglycerin
    add on
    organic_nitrate_vasodilator
    5-20 µg/min IV titrate • IV • continuous
    triggers: hypertensive_adhf_with_sbp_above_140_and_congestion
    Preload + modest afterload reduction for hypertensive ADHF; AVOID if SBP <100 or RV-predominant restrictive phase
    rxcui 4917
  • sacubitril_valsartan
    first line
    arni_neprilysin_inhibitor_arb
    24/26 mg PO BID (titrate to 49/51 then 97/103 BID) • PO • BID
    triggers: hfref_iron_overload_with_ef_below_40_and_sbp_above_100
    PIONEER-HF PMID 30403955 — in-hospital initiation; PARADIGM-HF — superior to ACEi for HFrEF; standard GDMT in iron-overload-related HFrEF; 36h washout from ACEi required
    rxcui 1656328
  • carvedilol
    first line
    beta_blocker_nonselective_alpha1
    3.125 mg PO BID titrate • PO • BID
    triggers: hfref_iron_overload_with_ef_below_40_and_euvolemia
    CAPRICORN PMID 11356436 + COPERNICUS PMID 11386262 — beta-blocker mortality benefit in HFrEF; standard 4-pillar GDMT; safe in iron-overload cardiomyopathy
    rxcui 20352
  • spironolactone
    first line
    mineralocorticoid_receptor_antagonist
    12.5-25 mg PO daily • PO • daily
    triggers: hfref_iron_overload_with_ef_below_35_and_k_below_5_and_egfr_above_30
    RALES PMID 10471456 — mortality benefit in HFrEF; monitor K + eGFR; renal dose-adjust; standard 4-pillar GDMT
    rxcui 9997
  • dapagliflozin
    first line
    sglt2_inhibitor
    10 mg PO daily • PO • daily
    triggers: hfref_iron_overload_with_ef_below_40
    DAPA-HF PMID 31535829 — mortality + HF hospitalization benefit; 4th pillar GDMT; safe in iron-overload cardiomyopathy; especially useful given DM comorbidity common in HH
    rxcui 1488564
  • empagliflozin
    first line
    sglt2_inhibitor
    10 mg PO daily • PO • daily
    triggers: adhf_in_hospital_initiation_per_empulse
    EMPULSE PMID 35347356 — in-hospital initiation safe + improves clinical benefit; alternative to dapagliflozin
    rxcui 1545653
  • deferasirox
    first line
    oral_iron_chelator
    21 mg/kg PO daily (Jadenu) — start lower 14 mg/kg if frail or eGFR borderline • PO • daily
    triggers: transfusion_dependent_iron_overload_with_cardiac_t2_below_20, hereditary_hemochromatosis_intolerant_of_phlebotomy_due_to_anemia_or_severe_hf
    EHA-EBMT 2022 consensus PMID 35139194 — first-line oral chelator with excellent cardiac penetration; monitor eGFR (renal toxicity), LFTs (hepatotoxicity), CBC (cytopenias); dose-adjust per renal function
    rxcui 614373
  • deferoxamine
    second line
    parenteral_iron_chelator
    40-60 mg/kg/day SC infusion 8-12 h/day 5-7 d/week • SC • continuous infusion
    triggers: severe_cardiac_iron_overload_t2_below_6ms_combination_chelation, deferasirox_intolerant_or_severe_renal_dysfunction, pediatric_or_pregnancy_chelation
    Historic standard chelator; reserved for severe iron overload requiring maximal cardiac iron mobilization; combination with deferiprone for cardiac iron clearance per Tanner PMID 17389272 TIC trials; poor compliance due to infusion burden
    rxcui 3131
  • deferiprone
    second line
    oral_iron_chelator_pyridinone
    75-100 mg/kg/day PO TID • PO • TID
    triggers: severe_cardiac_iron_overload_t2_below_10ms_for_cardiac_iron_clearance, combination_chelation_with_deferoxamine_for_severe_cases
    Pennell EHJ 2001 PMID 11713075 + 2006 PMID 16735649 — best cardiac iron clearance per T2* studies; risk of agranulocytosis (need WEEKLY CBC monitoring); often combined with deferoxamine for severe cardiac iron overload (T2* <6 ms) per Tanner PMID 17389272 TIC trials
    rxcui 11645
  • warfarin
    comorbidity specific
    vitamin_k_antagonist
    5 mg PO daily INR target 2-3 • PO • daily
    triggers: af_in_iron_overload_cardiomyopathy_with_chads_vasc_above_2, lv_thrombus_on_echo
    AC for AFib (very common in iron-overload — atrial iron deposition); AC for LV thrombus per AHA 2022 Class IIa; preferred over DOAC if cirrhosis with hepatic synthetic dysfunction
    rxcui 11289
  • apixaban
    comorbidity specific
    doac_factor_xa_direct
    5 mg PO BID (or 2.5 mg BID per dose-reduction criteria) • PO • BID
    triggers: af_in_iron_overload_cardiomyopathy_with_chads_vasc_above_2_and_no_severe_cirrhosis, lv_thrombus_warfarin_alternative
    ACC/AHA 2023 AFib PMID 38033089 — DOAC preferred over warfarin in most cases; AVOID if Child-Pugh C cirrhosis from iron-overload hepatopathy; ARISTOTLE PMID 21870978
    rxcui 1364430

outpatient playbook — drug actions (5)

  1. 1. continue 4-pillar GDMT at maximum tolerated
    rxcui 1656328
    sacubitril-valsartan + carvedilol + spironolactone + SGLT2i at goal • PO • as scheduled
    trigger: HFrEF maintenance
    2022 ACC/AHA HF Class I
  2. 2. continue deferasirox lifelong (transfusion-dependent or HH-intolerant)
    21 mg/kg PO daily titrate per ferritin trend • PO • daily
    trigger: transfusion-dependent or HH-intolerant of phlebotomy
    EHA-EBMT 2022 PMID 35139194
  3. 3. phlebotomy maintenance for HH
    2-6 phlebotomies per year per ferritin trend (target <50 ng/mL) • venesection • 2-6/year
    trigger: HH on phlebotomy maintenance phase
    EASL 2022 PMID 36064151 + AASLD 2011 PMID 21452290
  4. 4. continue AC if indicated
    rxcui 1364430
    apixaban or warfarin • PO • BID or daily
    trigger: AFib or persistent LV thrombus
    ACC/AHA 2023
  5. 5. manage comorbid endocrinopathies
    insulin for DM, levothyroxine for hypothyroidism, testosterone for hypogonadism • as appropriate • as scheduled
    trigger: endocrine deficits from iron deposition
    Endocrinology coordination per EASL 2022

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Ferritin >1000 ng/mL + transferrin saturation >50% + new HF symptoms (dyspnea, edema, fatigue) → suspect iron-overload cardiomyopathy; Known HFE C282Y homozygote or compound heterozygote with new HF symptoms — likely cardiac iron deposition; cardiac MRI T2* needed; Chronic transfusion-dependent patient (β-thalassemia major, sickle cell, MDS, aplastic anemia) with 20+ lifetime units + new HF — transfusional iron-overload cardiomyopathy.

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Acute HF — hemochromatosis / iron-overload cardiomyopathy** (cardio.acute-hf.hemochromatosis-iron-overload.v1).
Scope: Cardiac iron-overload cardiomyopathy decompensation: HH (HFE C282Y homozygote ~80%) or transfusional (chronic transfusion-dependent); restrictive then dilated phenotype; conduction system involvement; cardiac MRI T2* gold standard; phlebotomy (HH) or chelation (transfusion-dependent or HH-intolerant); GDMT 4-pillar standard

No severity triggers fired against current inputs.

Plan

Regimen axis: **Iron-overload cardiomyopathy ADHF — supportive ADHF + GDMT 4-pillar + PHLEBOTOMY (HH) or IRON CHELATION (transfusion-dependent or HH-intolerant); coordinate with hematology + hepatology + endocrinology (EASL 2022 PMID 36064151; AASLD 2011 PMID 21452290; EHA-EBMT 2022 PMID 35139194; Anderson PMID 11713075)**.
1. furosemide 40-80 mg IV (diuretic-naive); 2.5x outpatient PO dose IV if on chronic loop (DOSE-trial guided) IV q12h titrate (loop_diuretic, first line) — DOSE PMID 21366472 — high-dose IV bolus or continuous infusion equivalent; titrate to UOP + symptom resolution; transition to PO before discharge
2. nitroglycerin 5-20 µg/min IV titrate IV continuous (organic_nitrate_vasodilator, add on) — Preload + modest afterload reduction for hypertensive ADHF; AVOID if SBP <100 or RV-predominant restrictive phase
3. sacubitril_valsartan 24/26 mg PO BID (titrate to 49/51 then 97/103 BID) PO BID (arni_neprilysin_inhibitor_arb, first line) — PIONEER-HF PMID 30403955 — in-hospital initiation; PARADIGM-HF — superior to ACEi for HFrEF; standard GDMT in iron-overload-related HFrEF; 36h washout from ACEi required
4. carvedilol 3.125 mg PO BID titrate PO BID (beta_blocker_nonselective_alpha1, first line) — CAPRICORN PMID 11356436 + COPERNICUS PMID 11386262 — beta-blocker mortality benefit in HFrEF; standard 4-pillar GDMT; safe in iron-overload cardiomyopathy
5. spironolactone 12.5-25 mg PO daily PO daily (mineralocorticoid_receptor_antagonist, first line) — RALES PMID 10471456 — mortality benefit in HFrEF; monitor K + eGFR; renal dose-adjust; standard 4-pillar GDMT
6. dapagliflozin 10 mg PO daily PO daily (sglt2_inhibitor, first line) — DAPA-HF PMID 31535829 — mortality + HF hospitalization benefit; 4th pillar GDMT; safe in iron-overload cardiomyopathy; especially useful given DM comorbidity common in HH
7. empagliflozin 10 mg PO daily PO daily (sglt2_inhibitor, first line) — EMPULSE PMID 35347356 — in-hospital initiation safe + improves clinical benefit; alternative to dapagliflozin
8. deferasirox 21 mg/kg PO daily (Jadenu) — start lower 14 mg/kg if frail or eGFR borderline PO daily (oral_iron_chelator, first line) — EHA-EBMT 2022 consensus PMID 35139194 — first-line oral chelator with excellent cardiac penetration; monitor eGFR (renal toxicity), LFTs (hepatotoxicity), CBC (cytopenias); dose-adjust per renal function
9. deferoxamine 40-60 mg/kg/day SC infusion 8-12 h/day 5-7 d/week SC continuous infusion (parenteral_iron_chelator, second line) — Historic standard chelator; reserved for severe iron overload requiring maximal cardiac iron mobilization; combination with deferiprone for cardiac iron clearance per Tanner PMID 17389272 TIC trials; poor compliance due to infusion burden
10. deferiprone 75-100 mg/kg/day PO TID PO TID (oral_iron_chelator_pyridinone, second line) — Pennell EHJ 2001 PMID 11713075 + 2006 PMID 16735649 — best cardiac iron clearance per T2* studies; risk of agranulocytosis (need WEEKLY CBC monitoring); often combined with deferoxamine for severe cardiac iron overload (T2* <6 ms) per Tanner PMID 17389272 TIC trials
11. warfarin 5 mg PO daily INR target 2-3 PO daily (vitamin_k_antagonist, comorbidity specific) — AC for AFib (very common in iron-overload — atrial iron deposition); AC for LV thrombus per AHA 2022 Class IIa; preferred over DOAC if cirrhosis with hepatic synthetic dysfunction
12. apixaban 5 mg PO BID (or 2.5 mg BID per dose-reduction criteria) PO BID (doac_factor_xa_direct, comorbidity specific) — ACC/AHA 2023 AFib PMID 38033089 — DOAC preferred over warfarin in most cases; AVOID if Child-Pugh C cirrhosis from iron-overload hepatopathy; ARISTOTLE PMID 21870978

Setting playbook (outpatient) — Long-term cardiology + multidisciplinary surveillance: serial echo + cardiac MRI T2* annually for chelation response; ICD/transplant decision at 3-6 mo if EF persistently <35; phlebotomy maintenance (HH) or chelation maintenance (transfusion-dependent) lifelong; family genotype screening; endocrine + hepatic surveillance; HCC surveillance if cirrhosis
13. continue 4-pillar GDMT at maximum tolerated sacubitril-valsartan + carvedilol + spironolactone + SGLT2i at goal PO as scheduled — HFrEF maintenance (2022 ACC/AHA HF Class I)
14. continue deferasirox lifelong (transfusion-dependent or HH-intolerant) 21 mg/kg PO daily titrate per ferritin trend PO daily — transfusion-dependent or HH-intolerant of phlebotomy (EHA-EBMT 2022 PMID 35139194)
15. phlebotomy maintenance for HH 2-6 phlebotomies per year per ferritin trend (target <50 ng/mL) venesection 2-6/year — HH on phlebotomy maintenance phase (EASL 2022 PMID 36064151 + AASLD 2011 PMID 21452290)
16. continue AC if indicated apixaban or warfarin PO BID or daily — AFib or persistent LV thrombus (ACC/AHA 2023)
17. manage comorbid endocrinopathies insulin for DM, levothyroxine for hypothyroidism, testosterone for hypogonadism as appropriate as scheduled — endocrine deficits from iron deposition (Endocrinology coordination per EASL 2022)

Non-pharmacologic actions:
- Cardiac rehab maintenance phase
- Phlebotomy maintenance schedule (HH)
- Chelation adherence (transfusion-dependent)
- ICD evaluation at 3-6 mo if EF persistently <35
- Transplant referral if end-stage despite optimized GDMT + iron removal
- Family genotype screening (siblings + first-degree relatives)
- Diet counseling — no iron supplements, vitamin C megadoses, raw shellfish, abstinence if cirrhosis

AVOID / contraindication checks:
- Avoid_iron_supplements_in_iron_overload_cardiomyopathy (obvious; check inadvertent prescription including multivitamins)
- Avoid_high_dose_vitamin_c_supplementation_in_active_iron_overload (enhances iron absorption; modest dietary intake acceptable)
- Avoid_raw_shellfish_in_iron_overload (Vibrio vulnificus susceptibility from elevated transferrin saturation)
- Avoid_excessive_alcohol_in_hh_or_iron_overload (cirrhosis acceleration synergy; abstinence recommended if cirrhosis present)
- Avoid_nsaids_in_cirrhosis_from_iron_overload (renal + GI bleeding risks)
- Phlebotomy_contraindicated_in_transfusion_dependent_patients (cannot lose blood; chelation only)
- Phlebotomy_caution_with_severe_hf_or_anemia_or_hypotension (use chelation alternative)
- Deferasirox_monitor_egfr_q_monthly_renal_toxicity (dose reduce if eGFR <60; hold if eGFR <40)
- Deferasirox_monitor_lft_q_monthly_hepatotoxicity (hold if AST/ALT >5x ULN)
- Deferiprone_weekly_cbc_for_agranulocytosis_monitoring (1 2% incidence; hold if ANC <1500)
- Deferoxamine_audiovisual_screening_q_year (ototoxicity + retinal toxicity)
- Arni_avoid_concurrent_acei_or_arb_36h_washout (PIONEER HF + PARADIGM HF protocol; angioedema risk)
- Sglt2i_hold_if_dka_risk_or_aki (DAPA HF + EMPULSE protocols; especially relevant in HH related DM)
- Warfarin_avoid_active_bleeding_or_pregnancy (AHA 2022)
- Apixaban_avoid_severe_renal_impairment_egfr_below_25_and_severe_cirrhosis (drug label; Child Pugh C)

Monitoring

Regimen monitoring:
- continuous telemetry during admission for arrhythmia and av block surveillance
- daily weight io strict
- daily bmp for diuretic safety and gdmt initiation
- monthly egfr and lft during deferasirox therapy for renal and hepatic toxicity
- weekly cbc during deferiprone therapy for agranulocytosis monitoring
- q3mo ferritin trend target below 50 for hh or below 500 for transfusion dependent
- q3mo transferrin saturation trend
- annual cardiac mri t2star for chelation response assessment per anderson pmid 11713075
- annual liver mri r2 ferriscan if significant hepatic iron load
- q6 to 12 mo endocrine panel dm thyroid hypogonadism
- annual us and afp for hcc surveillance per aasld 2011 if cirrhosis
- icd eligibility evaluation at 3 to 6 mo if ef persistently below 35
- gdmt titration per strong hf protocol pmid 36356631
- family genotype screening first degree relatives per easl 2022
- transplant referral if end stage despite optimized gdmt and iron removal

Setting (outpatient) monitoring:
- Quarterly cardiology + serial echo
- Cardiac MRI T2* annually
- Q3mo ferritin + transferrin saturation
- Annual liver MRI R2 if significant hepatic iron
- INR if warfarin
- Holter for arrhythmia surveillance
- Annual US + AFP for HCC if cirrhosis

Follow-up plan: Cardiology follow-up at 1-2 wks + 3 mo + 6 mo + 12 mo; phlebotomy (HH) every 1-2 wks until ferritin <50, then maintenance 2-6/year; chelation (transfusion-dependent) lifelong with q3mo ferritin trend + annual T2* MRI; family genotype screening (siblings + first-degree relatives per EASL 2022); endocrinology follow-up (DM, hypogonadism, hypothyroidism); hepatology follow-up with annual US + AFP for HCC surveillance per AASLD 2011 (cirrhosis present); transplant evaluation if end-stage despite optimized GDMT + iron removal
- Close-out criterion: long-term plan + multidisciplinary follow-up + family screening documented

Monitoring phase: Continuous telemetry (arrhythmia + AV block surveillance); daily weight + I/O; daily BMP for diuresis safety + GDMT initiation + chelator monitoring (eGFR for deferasirox; CBC for deferiprone); ferritin q3mo on phlebotomy/chelation (trend down toward <50 for HH; <500 for transfusion-dependent); cardiac MRI T2* annually for chelation response; LFT + endocrine panel q6-12mo

Disposition

Current setting: outpatient — Long-term cardiology + multidisciplinary surveillance: serial echo + cardiac MRI T2* annually for chelation response; ICD/transplant decision at 3-6 mo if EF persistently <35; phlebotomy maintenance (HH) or chelation maintenance (transfusion-dependent) lifelong; family genotype screening; endocrine + hepatic surveillance; HCC surveillance if cirrhosis

Disposition criteria:
- Long-term continuation; if EF normalized at 6-12 mo with chelation response → consider GDMT taper trial under cardiology supervision; if persistent HFrEF → indefinite GDMT + ICD; cross-link to cardio.hf.core.v1 for chronic HFrEF management

Escalation triggers (move to higher acuity):
- Recurrent ADHF → admission
- Sustained VT → EP + ablation
- EF declining despite GDMT → advanced HF eval + transplant
- Chelator toxicity → hematology for dose adjustment or switch
- New endocrinopathy → endocrinology
- New HCC on surveillance → hepatology + oncology

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Cardiac MRI T2* <6 ms — imminent HF risk per Anderson PMID 11713075 + Wood PMID 18353963; aggressive combination chelation required (deferiprone + deferoxamine per Tanner PMID 17389272 TIC trials)
- [LIFE_THREATENING] Hemodynamic deterioration to SCAI C+ shock physiology (SBP <90 + lactate ≥2 + cool extremities + organ dysfunction) — iron-overload cardiomyopathy progression to shock
- [LIFE_THREATENING] High-grade AV block (Mobitz II, complete heart block) ± syncope from iron-deposition conduction system disease

Citations

- EASL 2022 hereditary hemochromatosis CPG (PMID 36064151) + AASLD 2011 hemochromatosis practice guideline (Bacon PMID 21452290) + EHA-EBMT 2022 iron chelation consensus (PMID 35139194) + Anderson EHJ 2001 cardiac MRI T2* gold standard (PMID 11713075) + 2022 ACC/AHA HF Guideline (PMID 35363499) [PMID:36064151](https://pubmed.ncbi.nlm.nih.gov/36064151/)
- Cited evidence (PMID 21452290) [PMID:21452290](https://pubmed.ncbi.nlm.nih.gov/21452290/)
- Cited evidence (PMID 35139194) [PMID:35139194](https://pubmed.ncbi.nlm.nih.gov/35139194/)
- Cited evidence (PMID 11713075) [PMID:11713075](https://pubmed.ncbi.nlm.nih.gov/11713075/)
- Cited evidence (PMID 16735649) [PMID:16735649](https://pubmed.ncbi.nlm.nih.gov/16735649/)

Last reconciled with current guidelines: 2026-05-15.
References
  • EASL 2022 hereditary hemochromatosis CPG (PMID 36064151) + AASLD 2011 hemochromatosis practice guideline (Bacon PMID 21452290) + EHA-EBMT 2022 iron chelation consensus (PMID 35139194) + Anderson EHJ 2001 cardiac MRI T2* gold standard (PMID 11713075) + 2022 ACC/AHA HF Guideline (PMID 35363499) — PMID:36064151
  • Cited evidence (PMID 21452290) — PMID:21452290
  • Cited evidence (PMID 35139194) — PMID:35139194
  • Cited evidence (PMID 11713075) — PMID:11713075
  • Cited evidence (PMID 16735649) — PMID:16735649